Wiskott-Aldrich Syndrome

Below you will find more information about Wiskott-Aldrich Syndrome from Medigest. If you believe that you are suffering from any of the symptoms of Wiskott-Aldrich Syndrome it is important that you obtain an accurate diagnosis from a medical professional to ensure that you obtain the correct medication or treatment for your condition. There are medical conditions that carry similar symptoms associated with Wiskott-Aldrich Syndrome and therefore the information provided by Medigest is offered as a guideline only and should never be used in preference to seeking professional medical advice. The information relating to Wiskott-Aldrich Syndrome comes from a third party source and Medigest will not be held liable for any inaccuracies relating to the information shown.

Definition

Wiskott-Aldrich syndrome otherwise known as the eczema-thrombocytopenia-immunodeficiency syndrome (Aldrich RA, Steinberg AG, Campbell DC (1954). "Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis and bloody diarrhea". Pediatrics 13 (2): 133-9) is a x-linked recessive disease that is characterized by eczema, thrombocytopenia of low platelet counts, immune deficiency and blood in diarrhea.

Diagnosis

Diagnosis is usually bases on clinical parameters especially in the blood film and low immunoglobulin levels. Usually the IgM levels for person with this condition is low and the IgA and IgE are elevated and paraproteins are observed (Radl J, Dooren LH, Morell A, Skvaril F, Vossen JM, Uittenbogaart CH (1976). "Immunoglobulins and transient paraproteins in sera of patients with the Wiskott-Aldrich syndrome: a follow-up study". Clin. Exp. Immunol. 25 (2): 256-63).

Treatment

Treatment of Wiskott-Aldrich syndrome would vary and greatly depends on the symptoms. However, in cases where there are low platelet counts, a splenectomy or platelet transfusion and blood transfusion for anemia is advised. Also intravenous immunoglobulins or IVIG are advised to boost the immune system of the person.

Symptoms and Signs

Its symptoms includes bruising thrombocytopenia or low platelet counts, small platelet size on bloody film, eczema, recurrent infections and possibility for autoimmune disorders and malignancies.

Causes

It is said to be inherited immune disorder caused by mutations in a gene that primarily affects the immune system.

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